Pemphigus hereditary ( epidermolysis bullosa )
Hereditary pemphigus - a large (over 20 options) a group of non-inflammatory diseases characterized by skin and mucous tendency to develop blisters, especially in the field of mechanical injuries, even minor (friction, pressure, intake of solid food).The disease is genetically heterogeneous, inherited as an autosomal recessive and an autosomal dominant manner.
main feature of the whole group of diseases is the appearance of blisters at the slightest mechanical injury, and not rarely and spontaneously.The disease begins with the first days of life, at least - at a later age.In the neonatal period all forms of the disease is more severe, frequent deaths.Perhaps struck by the entire skin, but in most cases the rash is located on the extremities.In a simple (nerubtsuyuschemsya) epidermolysis bullosa blisters on the skin and oral mucosa heal without scarring.Quite often, especially in the embodiment herpetiformis develops excessive keratinization in the skin of palms and soles.When heavier flowing epidermoly
prognosis for recovery unfavorable.
Treatment is aimed at eliminating the symptoms of the disease.Appointed by antibacterial preparations, to accelerate healing, vitamin E in large doses, vitamin A in normal dosages.In degenerative forms of the disease - phenytoin twice per day for 8-10 mg / kg body weight for children and 3.5 mg / kg - in adults.Prenatal diagnosis is carried out only in the presence of certain indications.